We report an uncommon case of simultaneous presence of hemophagocytic lymphohistiocytosis (HLH) with Hodgkin's lymphoma and tuberculosis in an immunocompetent male. In the absence of treatment HLH is frequently associated with fatal outcome, although spontaneous partial regression is also noted. The early diagnosis of HLH is critical to prevent end organ failure. There are defined criteria for diagnosis of HLH but visualization of hemophagocytosis in bone marrow smear or biopsy along with fever, splenomegaly, elevated ferritin & triglycerides and peripheral blood cytopenias are highly suggestive of HLH. The pathologist and clinician should be aware of this uncommon entity so that early diagnosis and institution of therapy can be done to prevent poor prognostic outcome.

The maiden description of hemophagocytic lymphohistiocytosis (HLH) was provided by Farquhar and Claireaux in 1952 as familial hemophagocytic reticulosis [1]. The authors characterized the disease as a histiocytic proliferation in solid organs along with the phagocytosis of cells of various hematopoietic lineage, thereby referring it as a hemophagocytic syndrome. The etiology is linked to a defect in inflammatory process resulting from an uncontrolled hypercytokinemia as a congenital or acquired defect in natural killer (NK) T–cell function of cytotoxic pathway. Although HLH is neither associated with any specific age groups nor has a gender predilection, the case-fatality rate is usually high accounting to over 95% in untreated children [2]. Even with currently recommended therapy, HLH is frequently associated with fatal outcomes, although spontaneous partial regression has also been reported. The early institution of therapy is critical to control the hypercytokinemia that otherwise may lead to end-organ failure and death [3]. In this report, we describe a case of a young male with tuberculosis that was diagnosed with HLH associated with Hodgkin's lymphoma.

The association between HLH and hematological malignancy including Hodgkin's lymphoma is well described. Approximately 1% of patients with Hodgkin's lymphoma develop HLH [6]. Tumors can cause HLH by excessive secretion of cytokines [7]. Our patient was on antitubercular regimen for an active history of mycobacterial tuberculosis, a plausible triggering factor for HLH.

Conclusion

Our report describes a case of HLH in a young Indian male. The classical hemophagocytosis seen in our case is critical in reaching a diagnosis of HLH. The simultaneous presence of typical granuloma, Hodgkin's cell, and hemophagocytosis is rare but important to note so that the complete diagnosis is made during the management of the patient.

Authors' Contributions

RS conceptualized the work, designed, wrote the manuscript. MK aided in conception, writing and literature search. GG contributed to the interpretation and final approval of manuscript.

Consent

Written informed consent was obtained for the publication of the images.